Slowly Progressive Amnesia without Dementia

This report concerns longitudin's investigations with comprehensive neuropsychologic's assessments and precise morphologic's, CT and MRI scan-based, and function's, SPECT scan-based brain imaging examinations of seven patients who had selective progressive amnesia on their initi's visit. Progressive loss of recent memory remained for sever's years the single most s'sient feature in six of them. The atrophy of the hippocamp's region was observed morphologic'sly, and dysfunction confined to the bilater's medi's tempor's lobe involving the hippocamp's area was demonstrated function'sly. These findings are consistent with recently reported atypic's cases of 'Szheimer's disease with unique neur-opathologic's changes, namely the presence of numerous neurofibrillary tangles and few plaques in the hippocamp's region only.     

Characteristics and Management of Residual or Slowly Recurred Intracranial Aneurysms

Objective

Residual aneurysm from incomplete clipping or slowly recurrent aneurysm is associated with high risk of subarachnoid hemorrhage. We describe complete treatment of the lesions by surgical clipping or endovascular treatment.

Methods

We analyzed 11 patients of residual or recurrent aneurysms who had undergone surgical clipping from 1998 to 2009. Among them, 5 cases were initially clipped at our hospital. The others were referred from other hospitals after clipping. The radiologic and medical records were retrospectively analyzed.

Results

All patients presented with subarachnoid hemorrhage at first time, and the most frequent location of the ruptured residual or recurrent aneurysm was in the anterior communicating artery to posterior-superior direction. Distal anterior cerebral artery, posterior communicating artery, and middle cerebral artery was followed. Repositioning of clipping in eleven cases, and one endovascular treatment were performed. No residual aneurysm was found in postoperative angiography, and no complication was noted in related to the operations.

Conclusion

These results indicate the importance of postoperative or follow up angiography and that reoperation of residual or slowly recurrent aneurysm should be tried if such lesions being found. Precise evaluation and appropriate planning including endovascular treatment should be performed for complete obliteration of the residual or recurrent aneurysm.     

En-plaque ventricular ependymoma.

An extremely unusual case demonstrating rare behaviour of an ependymoma is presented. The tumour spread in an en-plaque fashion and involved the ependymal lining of the entire ventricular system. The patient, a 56-year-old male, presented with symptoms of raised intracranial pressure and truncal ataxia. The management issues are analyzed on the basis of a brief literature survey.     

Progressive Intracranial Fusiform Aneurysms and T-Cell Immunodeficiency

In the pediatric population, intracranial fusiform aneurysms have been associated with human immunodeficiency virus/acquired immunodeficiency syndrome and rarely with opportunistic infections related to other immunodeficiencies. The HIV virus and other infectious organisms have been implicated in the pathophysiology of these aneurysms. We present a child with T-cell immunodeficiency but no evidence of human immunodeficiency virus or opportunistic intracranial infections that developed progressive bilateral fusiform intracranial aneurysms. Our findings suggest a role of immunodeficiency or inflammation in the formation of some intracranial aneurysms.     

Rapidly Progressive Intracranial Vasculopathy in Graft Versus Host Disease

After allogenic hematopoietic stem cell transplantation, cerebrovascular complications are uncommon, occurring in approximately 2%, and typically due to coagulopathy or infection. Graft versus host disease has been rarely reported to affect the central nervous system but these cases typically describe leukoencephalopathy, encephalitis, or perivascular infiltrates or vasculitis with subcortical ischemia or hemorrhage. We report a previously undescribed noninflammatory vasculopathy causing multifocal intracranial arterial occlusions and cerebral infarctions in a man following allogenic hematopoietic stem cell transplantation for chronic lymphocytic leukemia, which we propose to be a central nervous system manifestation of graft versus host disease.     

Unusual Presentation of Sarcoidosis: Solitary Intracranial Mass Lesion Mimicking a Glioma

We present a patient with sarcoidosis with an isolated intraparenchymal mass lesion that was similar to a glioma on magnetic resonance imaging. On fluid‐attenuated inversion recovery images, a small hyperintense signal change in the right uncus was observed. Three months later, enlargement of the abnormal signal lesion was observed. An initial diagnosis of glioma was made. A biopsy of the temporal lobe tumor was done. On histology, a noncaseating granulomatous inflammation consistent with neurosarcoidosis was diagnosed. Albeit rarely, we should consider the possibility of neurosarcoidosis in the differential diagnosis of isolated intraparenchymal mass lesion, when the mass is located beside the pia mater.     

Primary Diffuse Leptomeningeal Gliomatosis with Signs of Increased Intracranial Pressure and Progressive Meningeal Enhancement on MRI

A 21-year-old woman presented with a 5-month history of meningeal signs and evidence of intracranial hypertension and, as shown by magnetic resonance imaging (MRI), progressively more extensive meningeal enhancement, particularly within the spinal canal. Autopsy disclosed the presence of primary diffuse leptomeningeal gliomatosis with spinal cord predominance, possibly arising within heterotopic leptomeningeal glial tissue in the cervical region. No parenchymal primary lesion was identified. MRI with gadolinium appears to be the imaging modality of choice for the early detection of primary diffuse leptomeningeal neoplasia.     

Sarcoidosis presenting with stroke

A 25-year-old black man with sarcoidosis presented with transient ischemic attacks followed by sudden, persistent right hemiparesis. He gave a history of recent, recurrent lower motor neuron facial palsy. Computed tomography demonstrated an infarct in the left internal capsule. Chest x-ray film showed bilateral hilar and mediastinal lymphadenopathy and multiple opacities in the lung fields. Serum angiotensin converting enzyme concentration was raised, and a Kveim test was positive for sarcoidosis. Despite clear pathologic reports of cerebral vasculitis in neurosarcoidosis, the occurrence of stroke is extremely rare.     

Non‐Invasive Intracranial Pressure Monitoring with Transcranial Doppler in a Patient with Progressive Cerebral Venous Sinus Thrombosis

In many intracranial disease states, monitoring of intracranial pressure (ICP) is essential to evaluate response to the therapeutic measures as well as estimation of prognosis. Although, direct estimation of ICP is reliable, it is invasive and not possible in all patients. Transcranial Doppler (TCD) ultrasonography is a bedside and noninvasive technique that provides reliable and real‐time information about cerebral hemodynamics. We present a case of extensive and progressive cerebral venous sinus thrombosis in which TCD served as an excellent tool for monitoring ICP and the serial observations correlated closely with clinical status and ophthalmological findings.     

Slowly progressive aphemia--a case report]

A 69-year-old women was admitted to Tokyo Medical and Dental University Hospital because of slowly progressive difficulty in speech production over 8-years. On admission, her spontaneous speech was non-fluent, limited to one-syllable utterance, and severely efforty. But her visual and auditory comprehension was preserved. There was no significant general intellectual deterioration. Severe buccofacial apraxia, but no swallowing was observed. So we considered her difficulty in speech as aphemia. Three-dimensionally reconstructed surface MR image clearly showed severe atrophy in the posterior part of the left inferior frontal gyrus and the lower part of the left pre-central gyrus. The FDG-PET demonstrated a focal hypometabolism in the same region. The lesion in this area was suggested to be a cause of speech production difficulty in this case.     

Slowly progressive aphasia without generalized dementia

Six right-handed patients experienced a slowly progressing aphasic disorder without the additional intellectual and behavioral disturbances of dementia. The symptoms almost universally started in the presenium. The initial difficulty was an anomic aphasia in five of the patients and pure word deafness in the sixth. Continuous and gradual deterioration occurred in the five patients who presented with an anomic aphasia. They eventually experienced additional impairment of reading, writing, and comprehension. In four patients, other areas of comportment were not involved within the 5 to 11 years of follow-up. A more generalized state of dementia may have emerged in the other two patients, but only after 7 years of progressive and debilitating aphasia. Neurodiagnostic procedures were consistent with preferential involvement of the left perisylvian region. In one patient, cortical biopsy did not show any pathognomonic change; specifically, no neurofibrillary tangles, amyloid plaques, neuronal inclusions, or gliosis were seen. This condition may constitute a syndrome of relatively focal cerebral degeneration with a predilection for the left perisylvian region.     

Slowly progressive aphasia with striatal involvement

We have described four patients with slowly progressive aphasia with striatal involvement occurring at different stages in the course of the illness. There were two males and two females, and their ages ranged from 68 to 76 (mean: 72) years. The extrapyramidal signs included tremors, bradykinesia, rigidity, and focal dystonia, and one had weakness resembling stroke. There is a heterogeniety among patients with slowly progressive aphasia and the clinical features correspond to the functional anatomy of the areas involved rather than to the pathology.     

Sarcoidosis of the nervous system

Although sarcoidosis is rarely confined to the nervous system, any neurological features that do occur frequently happen early in the course of the disease. The most common neurological presentation is with cranial neuropathies, but seizures, chronic meningitis and the effects of mass lesions are also frequent. The diagnostic process should first confirm nervous system involvement and then provide supportive evidence for the underlying disease; in the absence of any positive tissue biopsy, the most useful diagnostic tests are gadolinium enhanced MRI of the brain and CSF analysis, although both are non-specific. The mainstay of treatment is corticosteroids, but these often have to be combined with other immunosuppressants such as methotrexate, hydroxychloroquine or cyclophosphamide. There is increasing evidence that infliximab is a safe treatment with good steroid sparing capacity.     

Sarcoidosis and the nervous system

Neurologic involvement occurs in 5 per cent of patients with sarcoidosis and is a presenting symptom in about half of those affected. The neurologic symptoms are due to a granulomatous process that mirrors that seen in the systemic form. Its diagnosis is usually established by the demonstration of sarcoidosis in other systems and supported by clinical, radiographic, and laboratory evidence. Any and all portions of the nervous system can be involved in either an acute or a chronic fashion. Early acute presentations tend to have excellent outcomes and the overall prognosis for the disease is good. The cranial nerves, especially the facial nerve, are most frequently involved and have a good prognosis. CNS involvement, usually in the form of a meningoencephalitis involving the hypothalamic region, is usually chronic and associated with poor prognosis. Peripheral nerve and muscle involvement is frequently asymptomatic. Although neuropathy or myopathy may present acutely, the usual presentation is subacute or chronic. There have been no controlled studies of therapy in neurosarcoidosis. Early implementation of corticosteroids remains the mainstay of treatment. Evidence for improvement with treatment is anecdotally reported in many cases, but progression of the disease also occurs despite therapy.